A continuum of communication supports for

Primary Progressive Aphasia Melanie Fried Oken, Ph.D., CCC/Sp & Aimee R. Mooney, M.S. CCC/Sp

October 2013

Oregon Speech-Language-Hearing Association

Learning Outcomes

1. Identify symptoms and assessment protocols for clinical diagnosis PPA

2. Describe 3 stages of SLP treatment for individuals with PPA

3. Explain the communication support approach for intervention:

1. Restorative 2. Compensatory 3. Environmental/partner training

SYMPTOMS OF PPA

What is PPA?

Definitions • PPA is a clinical dementia

syndrome caused by neurodegenerative disease

• Language function slowly declines due to neurodegenerative brain disease; eventually affecting additional cognitive, behavioral and functional domains

Characteristics • Average age of symptom

onset = mid-50’s to early 60’s (participation impact)

• Even representation of male & female

• Survival is ~ 7 years post diagnosis with highly variable prognosis

Diagnosis of PPA: Clinical Options

Neurological exam: Rate of onset, other illness, other symptoms, etiology: stroke vs. tumor vs. neurodegenerative vs. other Neuropsychological exam: Evidence of

language domain impairment in absence of other cog/beh deficits Speech/Language Pathology exam:

Assess language modalities and components

Diagnosis of PPA: Neuroanatomy

Neuroimaging: (MRI, CT, PET) – to rule out other diseases – MRI may indicate regions of atrophy

(evidence of L sided atrophy?) – PET may show physiological dysfunction

(hypometabolic activity?)

Diagnosis of PPA: Neuropathology

• The disease as seen under the microscope • Autopsy studies show PPA is associated

with a particularly underlying pathology • Advances in imaging and biofluid

biomarkers in the coming years should facilitate the diagnosis of specific pathology in PPA

• Why is this important? Information leads to more effective medication intervention

Clinical diagnostic criteria for PPA Mesulam, M. 2003; Gorno-Tempini et al, 2011

Inclusion Criteria 1. Language deficits emerge

slowly and progress 2. Language deficits most

prominent feature of exam 3. Aphasia is the identifiable and

principal cause of impairment in ADL, otherwise WNL

4. Aphasia is sole deficit (or most prominent) at onset and for initial stages of disease

Exclusion Criteria 1. Diseases other than

neurodegenerative can account for the symptoms: stroke, tumor

2. Psychiatric diagnosis accounts for symptoms

3. Predominant initial episodic memory, visuospatial and or executive function deficits occur early in the course

4. Prominent initial behavioral disturbance

The 3 variants of PPA

1. Nonfluent/agrammatic variant (PPA-G) – Resembles a degenerative expressive

aphasia 2. Semantic variant (PPA-S)

– Resembles a degenerative receptive aphasia 3. Logopenic variant (PPA-L)

– Resembles a degenerative conduction aphasia or mixed expressive-receptive aphasia

PPA-G: Nonfluent/Agrammatic Gorno-Tempini, Hillis, Wientraub, et al, Neurology , 2011

At least one of the following core features must be present: Agrammatism in language production Effortful halting speech with inconsistent speech sound

errors and distortions (apraxia of speech) At least 2 of 3 of the following other features must be present: Impaired comprehension of syntactically complex

sentences Spared single-word comprehension Spared object knowledge

VIDEOS of PPA-G:

PPA-S: Semantic Gorno-Tempini, Hillis, Wientraub, et al, Neurology , 2011

Both of the following core features must be present: Impaired confrontation naming Impaired single-word comprehension At least 3 of the following other features must be present: Impaired object knowledge, particularly for low

frequency or low familiarity items Surface dyslexia or dysgraphia Spared repetition Spared speech production

Videos of PPA-S

PPA-L: Logopenic Gorno-Tempini, Hillis, Wientraub, et al, Neurology , 2011 Both of the following core features must be present: Impaired single-word retrieval in spontaneous speech and

naming Impaired repetition of sentences and phrases

At least 3 of the following other features must be present: Speech errors in spontaneous speech and naming Spared single word comprehension and object knowledge Spared motor speech Absence of frank agrammatism

First symptoms described by patients or observed by care providers

• Anomia or “trouble thinking of or remembering specific words when talking or writing” (PPA-G and PPA-S).

• Slow, hesitant speech frequently punctuated by long pauses and filler words (PPA-G).

• Marked increase in speech errors (substitutions or distortions; PPA-G).

• Struggle for speech sounds, apraxia (PPA-G) • Difficulties understanding spoken words (PPA-S).

Progression of disease varies

• Yes/No confusion for responses • Apraxia of Speech (PPA-G)

oArticulatory groping with difficulty self correcting

oVowel distortions and inconsistent errors oIncreased frequency of articulatory errors

as word or phrase length increases • Written language often mimics spoken language • Mutism

ASSESSMENT PROTOCOLS

Speech/language pathology exam for PPA 1. Assess language competence:

articulation, fluency, syntax, grammar, word retrieval, repetition, comprehension, reading & writing

2. Assess language performance: demands of environment to & functional communication skills needed for different settings and situations

Domain Test instruments

Articulation Apraxia Battery for Adults, motor speech exam

Fluency BDAE seven point scale for phrase length, WAB fluency, Grammatical Competence & Paraphasias scale, clinician impression of fluency from spontaneous speech/picture descriptions

Syntax/grammar BDAE seven point scale for phrase length, WAB fluency, Grammatical Competence and Paraphasias scale, Northwestern Anagram Test, analysis of language samples

Word retrieval BNT, phonemic/category fluency tasks

Repetition WAB and BDAE repetition tasks (words, phrases, sentences)

Speech/language pathology exam in PPA Sapolsky, D., Domoto-Reilly, K., Negreira, A., Brickhouse, M.,McGinnis, S., Dickerson, B 2011

Domain Psycholinguistic test instruments Auditory comprehension

WAB and BDAE following commands tasks, BDAE Complex Ideational Material, PAL Sentence Comprehension, CYCLE Sentence-Picture Matching

Single word comprehension

BDAE Word Comprehension, WAB Auditory Word Recognition, CSB Category Comprehension, PALPA Spoken Word-Picture Matching, Peabody Picture Vocabulary Test

Reading & writing

WAB and BDAE written language tasks

BDAE: Boston Diagnostic Aphasia Examination; BNT: Boston Naming Test; CSB: Cambridge Semantic Battery; CYCLE: Curtiss-Yamada Comprehensive Language Evaluation –Receptive; PAL: Psycholinguistic Assessment of Language; PALPA: Psycholinguistic Assessments of Language Processing in Aphasia; WAB: Western Aphasia Battery

Speech/language pathology exam for PPA Sapolsky, D., Domoto-Reilly, K., Negreira, A., Brickhouse, M.,McGinnis, S., Dickerson, B 2011

Functional assessment tools

• Social Networks Inventory (Attainment Company)

• Aphasia Needs Assessment (Garrett & Beukelman, 2006) http://aac.unl.edu

• CETI: Communicative Effectiveness Index (Lomas et al., 1989)

Social Networks Blackstone & Berg, 2003

Client’s Circles: 1. Intimate partners

(family) 2.Good friends 3.Acquaintances 4.Employees 5.Strangers

PRINCIPLES OF TREATMENT

Treatment themes in PPA • Unlike chronic stroke, speech-language

abilities gradually decline in PPA • Start early & be proactive so person with

PPA can learn to use communication strategies and tools as soon as possible

• Consider all modalities (stimulation and compensation)

• Environmental/partner training from the beginning and throughout

• Adjust treatment as concomitant cognitive and motor difficulties develop

Consider the goal: Functional communication

• Maximize communication at each stage of disease

• Consider the individuals in the context of their environment and their multiple conversation partners

• Tailor treatment approach to current status; plan for likely progression and educate

Ideal model: Staged treatment approach

• Assess Treat Assess Treat • Goals evolve with symptom progression • 3 stages

I. Restorative II. Shift toward aided approaches III. Environmental support and partner training

Other contributing factors may affect treatment outcomes

• Changes in cognition – Memory (working memory and new learning) – Executive functions – Visuospatial processing

• Changes in behavior – Social and emotional changes – Disinhibition, apathy

• Motor changes – Weakness, incoordination – Limb apraxia – Fine motor dyscoordination

STAGES OF TREATMENT

Staging PPA treatment Fried-Oken, Rowland & Gibbons, 2010

Stage Treatment Partner Involvement I: RESTORATIVE Detectable language lapses with hesitations, dysfluencies and word-finding difficulties

Education; behavioral strategies to support conversation. Introduction of low tech AAC.

Behavioral training: -how to ask questions -provide choices -alter verbal and physical environment to support communication

II: COMPENSATORY Reduction in language use (circumlocutions, paraphasias, simplification, agrammatism)

Stage I + additionallow tech AAC. Transition to additional tools and techniques for multi-modal communication system, (mobile devices and SGD)

Device training: Partners learn message selection techniques and operations of each AAC tool.

III: ENVIRONMENTAL No functional language

Environmental Support Co-construction training: Partners lead successful interaction; support participation with multi-modal techniques.

STAGE I: RESTORATIVE

COMPENSATORY ENVIRONMENTAL

Naming treatment in Stage I PPA: • Semantic feature cuing (category,

function, location, associations, etc) • Phonetic cuing • Substitution (synonym or antonym) • Circumlocution; description • Picture sorting • Encourage self-cuing (Henry, ML, Rising, K., DeMarco, AT, Miller, BL,

Gorno-Tempini, & Beeson, PM. 2013)

STAGE II: RESTORATIVE

COMPENSATORY ENVIRONMENTAL

Stage II: A shift toward aided approaches Fried-Oken, Beukelman & Hux (2012)

• Expressive language is less efficient • Verbal participation in all activities

decreases • Telephone use decreases or is

avoided • Conversations become imbalanced

Communication Supports

Unaided Approaches (Natural modes)

• Speech • Vocalization • Gestures • Eye gaze • Body language • Sign language • Partner co-construction

Aided Approaches (Low tech and high tech tools)

• Paper and pencil • Communication books • Communication boards

and cards • Speaking computers • Talking typewriters • Speech generating

devices • Mobile technologies

Consider communication demands

– Different settings • Employment • Homes • Groups • Community events

– Different partners – Topics

• Familiar vs. unfamiliar

– Modes of communication • Telephone • Face to face, spontaneous • Written • Electronic

Low tech options

– Communication books – Communication passports – Photo albums – Pictures – Newspapers – Communication boards – Cards – Remnants – Written choice and continuum lines – Paper and pencil

High tech options • Dedicated speech generating devices • Mobile technology devices

User features to consider

• Previous experience with technology • Support for training • Partner’s experience with technology • Working memory abilities • Vision and hearing abilities; fine motor • Cognitive strategies and skills • Motivation

– “I bought this for mom to use.” – “She can’t seem to find the correct page.”

There is little empirical evidence that AAC helps people with

PPA-G with their daily expression. We only have case

studies and clinical descriptions.

The research challenge

Our research purpose To provide evidence that low

tech AAC (communication boards) and high tech AAC (mobile technology and apps) support adults with PPA during conversations.

To demonstrate that AAC

supports lexical access so that individuals can participate in daily activities as language skills decline.

Four studies

• Study 1: Do personalized low tech AAC boards in controlled conversations with research assistants improve expressive communication?

• Study 2: Do personalized, daily activities AAC boards used in conversations with frequent partner (spouse, child, caregiver) improve daily communication?

• Study 3: Is there generalization and maintenance of AAC over 6 months?

• Study 4: Does use of mobile technology for language support improve conversation in people with PPA?

Low tech AAC study (Fried-Oken & Rowland, submitted)

Method

• 20 individuals with PPA • 10 neurotypical adults and 20

individuals with AD • Personalized

communication boards • Trained in board use with

RA and with spouse • Scripted and natural

conversations; 6 with and 6 without AAC

PPA Results

• Targeted words produced at initial prompt significantly greater with AAC

• Number of questions posed by RA or spouses to elicit target words significantly reduced with AAC

Conversational board: My sports teams

Functional activities board

Interpretation of results • Low tech AAC provides

meaningful lexical support during structured conversations for people with PPA.

• Low tech AAC significantly reduces lexical scaffolding provided by the conversation partner.

• This approach should be part of a PPA treatment protocol.

Current mobile technology for PPA study: Sharing new information with/without AAC

Method • Justification: “ I can understand what

he is saying when I start the conversation. But when Jim comes up to me and wants to tell me something, and I don’t know the topic, I have no idea what he is talking about!”

• 4 adults with PPA (2 mild; 2 severe) • Completed 3 activities with RA • RAs populated 5 different layouts in

GoTalk Now app with photos & speech

• Barrier task: Tell your spouse what you did this afternoon

• 3 conversations with no AAC support; 3 conversations with AAC

Results • Significantly greater

report of gist with AAC than with no support

• For subjects with severe PPA: Total words spoken with AAC increased significantly compared to speech only condition;

• Specific app layout did not affect language performance

Visual Scene in GoTalk Now app

3 videos: (1) speech + gestures (2) speech + gestures + writing (3) speech + gestures + writing + GoTalk Now app

STAGE III: RESTORATIVE

COMPENSATORY ENVIRONMENTAL

Stage III: Emphasis on environmental modifications

• Engineering the environment • Communication partner training

Natural environmental supports • Pointing to weather pictures in newspaper

to indicate time of day • Pointing to framed boards on the family

picture wall at eye level • Using mail received from the bank to

indicate questions about finances • Flipping through pictures in photo book

during a family visit • Placing cue cards throughout environment • Remnant boxes

Partner training

• “Should I finish his sentences? Give him the words?”

• Support all forms of communication • Count to 10 in your head before expecting

a response • Speak in a quiet environment, establish

eye contact and reduce distractions • Set up a way to “come back to that later.”

www.reknewprojects.org -> Primary Progressive Aphasia -> Communication supports

www.reknewprojects.org -> Primary Progressive Aphasia -> Communication supports

Templates: 1 and 4-topic boards (Microsoft Publisher)

1-topic board Placed on file folder Box sizes can be manipulated

4-topic board Placed on file folder Box sizes can be manipulated

Documenting successful outcomes for people with PPA

• Stability = progress • Comprehension and production in any

modality is acceptable

Poll everywhere

Book and website references for AAC • www.aac-rerc.com: Research to practice AAC for

PPA webinar (AAC Rehabilitation Engineering Research Center)

• http://memory.ucsf.edu • www.brain.northwestern.edu (Cognitive Neurology

and Alzheimer’s Disease Center) • Simmons-Mackie, King & Beukelman (2013) Brookes

Publishing

Webinar Series for PPA: http://www.brain.northwestern.edu/ about/events/webinar.html

Dr. Lawrence Albert Memorial Webinar Series for SLPs treating Primary Progressive Aphasia

The Northwestern CNADC partnered with the National Aphasia

Association and the Association for Frontotemporal Degeneration to develop a series of webinars for Speech and Language Pathologists (SLPs) who treat people with primary progressive aphasia (PPA). This collaboration was formed to improve understanding and treatment approaches for SLPs who have had limited experience with PPA in their training or career. The live events were held in the 2013 Spring/Summer.

The archived webinars are available on demand and are complimentary to

SLPs, other professionals and families who wish to learn more about primary progressive aphasia and speech therapy.

Archived Webinars

"The ABCs of PPA for SLPs: Clinical Attributes, Biology and Care of Primary Progressive Aphasia". Presented by Sandra Weintraub, PhD, of the Northwestern CNADC. To view an archived recording of this presentation, please click here. For a PDF version of the slides, please click here.

"Treatment for Persons with PPA: An Adaptable Communication Support

Approach". Presented by Melanie Fried-Oken, PhD, CCC-SLP of Oregon Health and Science University and Maya Henry, PhD, CCC-SLP of University of California San Francisco. To view an archived recording of this presentation, please click here. For a PDF version of the slides, please click here.

"Living with Primary Progressive Aphasia: Challenges Experienced by

PPA Patients and Families and How SLPs Can Help!" Presented by Darby Morhardt, MSW, LCSW of the Northwestern CNADC and Jamie Reilly, PhD, CCC-SLP of University of Florida. To view an archived recording of this presentation, please click here. For a PDF version of the slides, please click here.

References Fried-Oken, M., Beukelman, D., & Hux, K. (2012). Current and future AAC research

considerations for adults with acquired cognitive and communication impairments. Assistive Technology, 24, 56-66.

Fried-Oken, M., Rowland, C., & Gibbons, C. (2010). Providing augmentative and alternative communication treatment to persons with progressive nonfluent aphasia. Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders, 20, 21-25.

Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. F., et al. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76(11), 1006-1014.

Henry, M. L., Rising, K., Demarco, A. T., Miller, B. L., Gorno-Tempini, M. L., & Beeson, P. M. (2013). Examining the value of lexical retrieval treatment in primary progressive aphasia: Two positive cases. Brain and Language.

Lomas, J., Pickard, L., Bester, S. Elbard, H., Finlayson, A., & Zoghaib, C. (1989). The communicative effectiveness index: Development and psychometric evaluation of a functional communication measure for adult aphasia. JSHD, 54. 113-124.

Mesulam, M. M. (2013). Primary progressive aphasia and the language network: The 2013 H. houston merritt lecture. Neurology, 81(5), 456-462.

Mesulam, M. M. (2001). Primary progressive aphasia. Annals of Neurology, 49(4), 425-432. Sapolsky, D., Domoto-Reilly, K., Negreira, A., Brickhouse, M., McGinnis, S., & Dickerson,

BC. (2011). Monitoring progression of primary progressive aphasia: current approaches and future directions. Neurodegen. Dis Manage. 1(1).

Disclosure statement

• We have no financial or nonfinancial interest in any organization whose products or services are described, reviewed, evaluated or compared in the presentation.

www.aac-rerc.com and

www.reknewprojects.org

Portions of the work in this presentation have been funded in part by the National Institute on Disability and Rehabilitation

Research (NIDRR), U.S. Department of Education under Grants #H133E030018 and #H133G080162

Pennsylvania State University subcontract from the Hintz

Family Endowment to OHSU